Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
Images in Dermatology
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Media and news
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Images
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Snippets
Special Article
Specialty Interface
Studies
Study Letter
Study Letters
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
Therapy Letters
View Point
Viewpoint
What’s new in Dermatology
Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
Images in Dermatology
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Media and news
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Images
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Snippets
Special Article
Specialty Interface
Studies
Study Letter
Study Letters
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
Therapy Letters
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF

Translate this page into:

Observation Letter
89 (
1
); 106-109
doi:
10.25259/IJDVL_596_2021
pmid:
36331851

Metastatic anaplastic large cell lymphoma of the omentum presenting as an ulcerated nodule on the back

Department of Dermatology, Universidad Nacional de Córdoba, Argentina
Department of Pathology, Universidad Nacional de Córdoba, Argentina

Corresponding author: Dr. Jimena Agostina Miranda, Department of Dermatology, Hospital Nacional de Clínicas, Santa Rosa 1564, X5000 ETF, Córdoba, Argentina, jam.dermatologia@gmail.com

Licence
This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: Miranda JA, Elías MB, Mazzotta MM, Zalazar ÉV. Metastatic anaplastic large cell lymphoma of the omentum presenting as an ulcerated nodule on the back. Indian J Dermatol Venereol Leprol 2023;89:106-9.

Sir,

Anaplastic lymphoma kinase (ALK)-negative anaplastic large-cell lymphoma is a rare peripheral T-cell non-Hodgkin lymphoma that belongs to the group of CD30-positive lymphoproliferative disorders and tends to affect adults over the age of 40 with a bad prognosis.1-3 It involves the lymph nodes and extranodal sites, but the involvement of the omentum as the primary site is rare. In ALK-negative anaplastic large-cell lymphoma patients, skin is one of the most common sites of spread. Secondary cutaneous lesions may be difficult to differentiate from primary cutaneous anaplastic large cell lymphoma.1,4

Herein, we describe an unusual case of a 67-year-old woman who presented with an ulcerated lesion on the back as secondary cutaneous involvement of primary ALK-negative anaplastic large-cell lymphoma of the omentum.

A 67-year-old woman presented with a two-week history of a rapidly growing painful ulcerated lesion on her back. She also complained of sporadic abdominal pain and weight loss for four months. Physical examination revealed ulcerated violaceous nodule measuring 3 cm in diameter in the lumbar region [Figure 1a]. Ultrasound of soft tissue showed a nodular mass with cystic-necrotic areas. Differential diagnosis of tuberculosis and neoplasia were initially considered. The lesion was biopsied for microbiological culture and histological examination. Bacterial, mycobacterial, and fungal cultures were negative. Haematoxylin and eosin staining showed dermal perivascular infiltration of large atypical lymphoid cells extending into the subcutaneous fat [Figures 1b and c ]. Immunohistochemistry was positive for CD3, CD4, CD7, CD8, CD30 (uniform and strong) [Figure 1d], and negative for CD5, CD56, epithelial membrane antigen, Epstein-Barr virus and ALK-1 [Figure 1e]. The expression of Ki-67 was 80% positive. The diagnosis of primary systemic anaplastic large cell lymphoma with skin involvement was considered. A computed tomography scan of the abdomen and pelvis revealed a poorly defined mass with soft tissue density in the omentum, in contact with the transverse colon and free fluid in the cul-de-sac [Figure 2a]. Laboratory workup was notable for an erythrocyte sedimentation rate of 124 mm/h and C-reactive protein of 14.9 mg/dL. Blood routine examination showed white blood cell count at 19,500/μL, haemoglobin count at 10.4 g/dL and platelet count at 745,000/μL. Serologic tests for HIV, hepatitis C, hepatitis B, human T-lymphotropic virus type 1 (HTLV-I) and Epstein-Barr virus were negative. She underwent an exploratory laparotomy for resection of the abdominal mass. Histology with immunohistochemical staining showed the same features as the previous skin biopsy [Figures 2b to d]. The diagnosis of primary ALK-negative anaplastic large cell lymphoma of the omentum with cutaneous metastases was made. The bone marrow biopsy result was normal. Although she was treated with four cycles of cyclophosphamide, adriamycin, vincristine, and prednisone regimen, her clinical condition worsened with decreasing mental status. Magnetic resonance imaging of the brain revealed multiple brain metastases. She died three months later due to disease progression.

A round ulcerated violaceous nodule in the lumbar region
Figure 1a:
A round ulcerated violaceous nodule in the lumbar region
Dense dermal and hypodermal infiltrate of large atypical lymphocytes (haematoxylin and eosin, ×4)
Figure 1b:
Dense dermal and hypodermal infiltrate of large atypical lymphocytes (haematoxylin and eosin, ×4)
Large pleomorphic hyperchromatic lymphoid cells with prominent nucleoli (haematoxylin and eosin, ×400)
Figure 1c:
Large pleomorphic hyperchromatic lymphoid cells with prominent nucleoli (haematoxylin and eosin, ×400)
The atypical lymphocytes stained positive for CD30 (×100)
Figure 1d:
The atypical lymphocytes stained positive for CD30 (×100)
The atypical lymphocytes stained negative for ALK (×400)
Figure 1e:
The atypical lymphocytes stained negative for ALK (×400)
Abdominal CT image shows a tumour in the omentum, in contact with the transverse colon (white arrow)
Figure 2a:
Abdominal CT image shows a tumour in the omentum, in contact with the transverse colon (white arrow)
Omentum: adipose tissue with dense and diffuse infiltrate of large atypical lymphoid cells (haematoxylin and eosin, ×10)
Figure 2b:
Omentum: adipose tissue with dense and diffuse infiltrate of large atypical lymphoid cells (haematoxylin and eosin, ×10)
Omentum: CD30-positive immunostaining (×100)
Figure 2c:
Omentum: CD30-positive immunostaining (×100)
Omentum: ALK-negative immunostaining (×50)
Figure 2d:
Omentum: ALK-negative immunostaining (×50)

ALK-negative anaplastic large cell lymphoma is an aggressive CD30-positive T-cell neoplasm morphologically indistinguishable from its ALK-positive counterpart.1 ALK-positive refers to the presence of a protein called ALK. These tumours have typically favourable outcomes. In contrast, ALK-negative anaplastic large-cell lymphoma is characterized by the lack of expression of ALK and this failure to express ALK protein has been shown to portend a worse prognosis.2,3

The exact prevalence of this tumour is unknown. It tends to affect patients over 40 years, unlike ALK-positive anaplastic large-cell lymphoma that occurs in children and young adults. A male predominance with a male:female ratio of 1.5:1 is reported.4

ALK-negative anaplastic large-cell lymphoma primarily involves peripheral, mediastinal or abdominal lymph nodes, but the disease can present at any site, including the central nervous system, oral cavity, bladder, pancreas and liver.1,5 However, to the best of our knowledge primary involvement of the omentum has not been reported.

In ALK-negative anaplastic large-cell lymphoma patients, the skin, liver and lung are the most common sites for secondary spread, compared with soft tissue and bone in ALK-positive anaplastic large-cell lymphoma.1 Of note, primary cutaneous anaplastic large cell lymphoma is typically negative for ALK and can be clinically indistinguishable from secondary skin involvement by systemic ALK-negative anaplastic large-cell lymphoma. However, this subtype rarely disseminates.6

It is important to differentiate cases of systemic ALK-negative anaplastic large-cell lymphoma from ALK-negative primary cutaneous anaplastic large-cell lymphoma, as the prognosis varies greatly. The primary cutaneous anaplastic large-cell lymphoma has a very favourable 5-year overall survival of 90% as compared to 49% for systemic ALK-negative anaplastic large-cell lymphoma.3

Cutaneous clinical presentation in systemic ALK-negative anaplastic large-cell lymphoma includes nodules, plaques, and ulcerated lesions without a predominant localization. Rieger et al., reported a case of an ALK-negative systemic anaplastic large-cell lymphoma presenting as a violaceous plaque on the scalp of a 79-year-old man.3 Long et al., described a 47-year-old man with anaplastic large-cell lymphoma cutaneous metastases presenting as multiple light pink annular papules and nodules with central ulceration on the face and neck.7

Yang et al., reported one case of systemic ALK-negative anaplastic large-cell lymphoma with secondary ulcerated cutaneous lesions and an aggressive progression although treated by cyclophosphamide, adriamycin, vincristine and prednisone.8 Alcalá et al., confirmed the diagnosis of breast implant-associated ALK-negative anaplastic large-cell lymphoma in old women with breast cutaneous lesions, treated by cyclophosphamide, adriamycin, vincristine, and prednisone chemotherapy without response.9

In our case, we excluded the diagnosis of primary cutaneous anaplastic large-cell lymphoma with metastasis to the omentum since the patient presented with skin lesion four months after the onset of abdominal pain. In addition, the disease progressed aggressively with metastases to other organs leading to her death in a few months.

Biopsies are challenging as histological findings can emulate other hematologic and solid malignancies.10 Immunohistochemical analysis is necessary for definitive diagnosis. Tumour cells can express diverse surface receptors including CD3, CD4, CD7, CD8, CD43, CD56 and EMA, but a diffuse and strong positive staining for CD30 is characteristic. ALK immunohistochemistry is negative by definition.11 In the present case, the tumour showed diffuse expression of CD30, CD3, CD4, CD7 and CD8, and negative for ALK stain, thus concluding the diagnosis of ALK-negative anaplastic large-cell lymphoma.

Chemotherapy (cyclophosphamide, doxorubicin, vincristine, prednisone) is the first-line treatment of ALK-negative anaplastic large-cell lymphoma. It may be followed by autologous stem cell transplantation after the first complete remission.12 However, recurrences are frequent.1 Although our patient underwent treatment with cyclophosphamide, adriamycin, vincristine, and prednisone regimen, the disease rapidly progressed.

In conclusion, this case emphasises the involvement of skin as a visible and diagnostic sign of systemic neoplasia. Moreover, in this clinical presentation, it is important to differentiate primary from secondary ALK-negative anaplastic large-cell lymphoma, as the prognosis can vary significantly. Diagnosis of such cases represents a challenge and may be based upon a combination of clinical behaviour, pathological morphology and immunophenotype.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Declaration of patient consent

Patient’s consent is not required as the patient’s identity is not disclosed or compromised.

References

  1. , , , . Anaplastic large cell lymphoma, ALK-negative. Crit Rev Oncol Hematol. 2013;85:206-15.
    [CrossRef] [PubMed] [Google Scholar]
  2. , , , , , , et al. Morphologic features of ALK-negative anaplastic large cell lymphomas with DUSP22 rearrangements. Am J Surg Pathol. 2016;40:36-43.
    [CrossRef] [PubMed] [Google Scholar]
  3. , , , . ALK-negative systemic intravascular anaplastic large cell lymphoma presenting in the skin. J Cutan Pathol. 2011;38:216-20.
    [CrossRef] [PubMed] [Google Scholar]
  4. , . Anaplastic large cell lymphomas: ALK positive, ALK negative, and primary cutaneous. Adv Anat Pathol. 2015;22:29-49.
    [CrossRef] [PubMed] [Google Scholar]
  5. , , . ALK Negative Anaplastic Large Cell Lymphoma. . Treasure Island (FL): StatPearls Publishing; StatPearls [Internet] [cited 2021 Nov 10]. Available from:
    [Google Scholar]
  6. , , , . The t(2;5) chromosomal translocation is not a common feature of primary cutaneous CD30+ lymphoproliferative disorders: Comparison with anaplastic large-cell lymphoma of nodal origin. Blood. 1996;87:3437-41.
    [PubMed] [Google Scholar]
  7. , , , , , , . Recurrent systemic anaplastic large cell lymphoma: Rapid onset and resolution of cutaneous metastases. JAAD Case Rep. 2020;6:124-7.
    [CrossRef] [PubMed] [Google Scholar]
  8. , , , , , , . Cutaneous anaplastic large-cell lymphoma should be evaluated for systemic involvement regardless of ALK-1 status: Case reports and review of literature. Am J Clin Dermatol. 2011;12:203-9.
    [CrossRef] [PubMed] [Google Scholar]
  9. , , , , , . Skin involvement as the first manifestation of breast implant-associated anaplastic large cell lymphoma. J Cutan Pathol. 2016;43:602-608.
    [CrossRef] [PubMed] [Google Scholar]
  10. , , , , . Anaplastic large cell lymphoma: One or more entities among T-cell lymphoma? Hematol Oncol. 2009;27:161-70.
    [CrossRef] [PubMed] [Google Scholar]
  11. , , , , , , et al. ALK- anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: Report from the International Peripheral T-Cell Lymphoma Project. Blood. 2008;111:5496-504.
    [CrossRef] [PubMed] [Google Scholar]
  12. , . The biology and management of systemic anaplastic large cell lymphoma. Blood. 2015;126:17-25.
    [CrossRef] [PubMed] [Google Scholar]

Fulltext Views
4,036

PDF downloads
2,602
View/Download PDF
Download Citations
BibTeX
RIS
Show Sections