|Year : 2017 | Volume
| Issue : 6 | Page : 585-588
Primary malignant melanoma of the gastroesophageal junction with plasmacytoid features and reactivity to cd138: A case report and review
Rajeshwari K Muthusamy1, Sangita S Mehta1, Albina Venus1, Bharath Rangarajan2
1 Department of Pathology, Kovai Medical Center and Hospital, Coimbatore, Tamil Nadu, India
2 Department of Medical Oncology, Kovai Medical Center and Hospital, Coimbatore, Tamil Nadu, India
|Date of Submission||07-Dec-2016|
|Date of Acceptance||30-Jan-2017|
|Date of Web Publication||17-Jan-2018|
Dr. Rajeshwari K Muthusamy
Department of Pathology, Kovai Medical Center and Hospital, Coimbatore - 641 014, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Melanoma of mucosal sites is rare and is extremely rare in esophagus. Melanoma is known for its histological diversity mimicking any other malignancies with poor prognosis. Clinical presentation may be similar to other gastroesophageal junction malignancies with obstructive symptoms/bleeding with pigmented polypoid mass on endoscopy and gross examination. However, metastasis from elsewhere is to be considered in the absence of melanocytosis in the adjacent squamous epithelium. Immunohistochemistry with a panel of markers is necessary in amelanotic lesions, poorly differentiated morphology, and small biopsy specimens. We report a case of melanoma with plasmacytoid features in the gastroesophageal junction diagnosed in a small biopsy with immunohistochemical findings and follow-up esophagogastrectomy.
Keywords: CD138, esophagus, melanoma, mucosal, plasmacytoid
|How to cite this article:|
Muthusamy RK, Mehta SS, Venus A, Rangarajan B. Primary malignant melanoma of the gastroesophageal junction with plasmacytoid features and reactivity to cd138: A case report and review. Med J DY Patil Univ 2017;10:585-8
|How to cite this URL:|
Muthusamy RK, Mehta SS, Venus A, Rangarajan B. Primary malignant melanoma of the gastroesophageal junction with plasmacytoid features and reactivity to cd138: A case report and review. Med J DY Patil Univ [serial online] 2017 [cited 2020 Feb 26];10:585-8. Available from: http://www.mjdrdypu.org/text.asp?2017/10/6/585/223364
| Introduction|| |
Primary mucosal malignant melanoma is rare, comprising 1% of all melanomas. Mucosal melanomas in the gastrointestinal tract are usually from anorectal region. Primary malignant melanoma of the esophagus (PMME) is extremely rare, constitutes about 0.1%–0.2% of the primary esophageal malignancies and 0.5% of all melanomas. Melanoma is known for its histological diversity mimicking other malignancies such as lymphoma, sarcoma, and poorly differentiated carcinoma on microscopic evaluation. As in any other site, a broad panel of immunohistochemical markers is necessary to clinch the diagnosis. Malignant melanoma with plasmacytoid features are extremely rare with very few case reports, show aberrant expression of CD138 in addition to melanocytic markers. We report a case of esophageal melanoma with features resembling plasmacytoid cells and presence of CD138 expression.
| Case Report|| |
A 62-year-old female presented with malena and progressive dysphagia for 2 months. She had a history of retrosternal discomfort, early satiety, and exertional dyspnea for 2 weeks. She was febrile at presentation. She did not have any significant past medical or surgical history. On physical examination, she was pale. Pedal edema was easily recognizable. There was no palpable lymphadenopathy. Upper airway examination is normal. Blood investigations revealed severe anemia with hemoglobin of 3.4 g/dl, increased reticulocyte count (4.2%), low iron, Vitamin B12 levels, and negative direct Coombs test. Motion occult blood was positive.
Contrast-enhanced computerized tomography of the chest and abdomen revealed eccentric wall thickening in the right lateral wall of esophagus for a length of 4.4 cm [Figure 1]a and [Figure 1]b. The lesion extends along the lesser curvature of stomach with heterogeneously enhancing polypoidal component of size 5.8 cm × 4.2 cm × 6 cm. Enlarged perigastric nodes were seen along the lesser curvature, the largest measured of size 3.4 cm [Figure 1]c. The other visualized organs were normal.
|Figure 1: (a and b) Contrast enhanced computerized tomography images showing lesion along the lesser curvature of stomach and right lateral wall of esophagus (red arrow) and (c) large perigastric lymph node (yellow arrow)|
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Gastroscopy was performed, revealed a polypoidal lesion starting at 32 cm of esophagus, extending into the fundus of stomach. Biopsy of the lesion showed a tumor comprising of sheets of cells with enlarged nuclei, and prominent nucleoli [Figure 2]a and [Figure 2]b. Few of the cells had eccentric nuclei resembling signet ring cells of adenocarcinoma [Figure 3]. Immunohistochemical analysis with a panel of markers using pankeratin, vimentin, S-100, CD45, CD138, CD20, CD79a, CD117, human melanoma black 45 (HMB45), Melan-A, p63, CD56, and Ki-67 done. The tumor cells showed strong positivity to vimentin [Figure 4]a, S-100 [Figure 4]b, HMB45 [Figure 4]c, Melan-A [Figure 4]d, focal reactivity to CD138 [Figure 5]a, and high Ki-67 of 45% [Figure 5]b. With the above histomorphology and immunohistochemical findings, a diagnosis of plasmacytoid melanoma of the esophagus was made. There was no skin, anal mucosal or orbital lesions on examination.
|Figure 2: (a) Small biopsy showing gastric mucosa and tumor fragment with sheet of cells (H and E, ×10). (b) High power view showing large tumor cells with prominent nucleoli (H and E, ×40)|
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|Figure 3: Sheets of large tumor cells with eccentric nuclei and prominent nucleoli (H and E, ×40)|
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|Figure 4: The tumor cells show positivity to vimentin (a), S-100 (b), human melanoma black 45 (c) and Melan-A (d)|
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|Figure 5: The tumor cells with CD138 positivity (a), and high proliferation index of 45% (b)|
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The patient underwent radical esophagogastrectomy with two-field lymphadenectomy. Gross examination revealed a polypoid soft grayish tumor in the esophagogastric junction, projecting as a large polypoid growth into the stomach, involving the lesser curvature, measured of size 8.5 cm × 6.3 cm × 2.8 cm. Another blackish lesion of size 2 cm was seen proximal to the tumor in the esophagus, cut section of which was black and cystic. A large lymph node with tumor involvement was seen in the antropyloric region [Figure 6]. Histology of the polypoid tumor and black cystic lesion showed plump cells in sheets with foci of necrosis. Cells with melanin pigment, increased mitosis, and lymphovascular emboli were present. Esophageal squamous epithelium in the tumor proximity showed melanocytosis and tumor arising from the area with junctional activity, establishing esophagus as the primary site. Four of the 23 lymph nodes isolated revealed tumor metastasis. There was no evidence of extracapsular spread. The patient declined further adjuvant immunotherapy and continues to be on observation after surgery.
|Figure 6: Gross specimen of esophagogastrectomy showing grayish polypoid tumor in the distal most esophagus and esophagogastric junction with extension into the lesser curvature of stomach and grossly involved nodgrossly involved nodgrossly involved nod grossly involved node|
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| Discussion|| |
Primary mucosal malignant melanoma of the esophagus was described by Baur in 1906. PMME is frequently reported in the distal part of the esophagus., PMME in the esophagogastric junction  is extremely rare with very few case reports. It occurs in sixth to seventh decade of life with male preponderance, extremely poor prognosis, and rapidly fatal course.,, PMME of the esophagogastric junction must have arisen from the distal part of esophagus and extend to involve the esophagogastric junction and stomach.
Imaging confirms the presence of a mass lesion and the presence or absence of nodal, liver, and lung metastasis. However, the diagnosis of PMME is purely histological, with almost all cases requiring immunohistochemistry for definitive diagnosis. PMME has to be kept as a differential diagnosis in poorly differentiated malignancies of the lower esophagus and esophagogastric junction, in addition to usual malignancies of the site. The primary site of melanoma as esophagus depends on the presence of melanocytosis in the adjacent squamous epithelium, which is difficult to get in small biopsies and by the absence of skin lesions by clinical examination.,,
PMME usually presents as a polypoid growth with partial gray to black pigmentation. Histology show sheets of tumor cells with prominent nucleoli, cytoplasmic melanin granules in some, and atypical melanocytes in the adjacent squamous epithelium.,,, Our patient also showed similar features on gross and microscopy with foci showing cells with eccentric nuclei suggesting plasmacytoid features with positivity to CD138 and lymph node metastasis. In the absence of melanin granules, immunohistochemistry is essential in establishing the diagnosis.
The tumor shows strong reactivity to S-100, vimentin, HMB45, and Melan-A.,, Variable positivity to CD138 can be seen in melanomas of usual morphology and with plasmacytoid features. Reactivity to HMB45 and Melan-A in the presence of CD138 helps to exclude plasmablastic lymphoma [Table 1].
|Table 1: Histological and immunohistochemical features in various malignancies of the esophagogastric junction|
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| Conclusion|| |
Melanoma of the esophagogastric junction is extremely rare, pose difficulty in diagnosis especially in small biopsies. A high index of suspicion, use of immunohistochemical markers and clinical correlation is necessary to clinch the diagnosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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